Our daughter Hailey was diagnosed with Leukodystrophy in 2011, a terminal brain disease. There is no cure or treatment. In January of 2015 she had a Pacemaker placed. There are medical bills, prescriptions not covered by insurance, Dr. Appointments out of state, and surprise expenses that would take a toll on any family. One way to share our story is to share jewelry we created together. There are many places we want to take her.
Each necklace signifies a Hug for Hailey.

Sunday, November 25, 2012

Our Story Continues...back in the hospital to SLC

When we arrive at the local ER they see that Hailey is pretty unresponsive and so so pale. In the triage she is vomiting, has a 104 fever and has diarrhea. They rehydrate her with a bolus IV and draw blood. They also give her rectal tylenol, and antibiotics in case this is an infection. They take our history, even though they have everything on file and tell us to get comfy, because they will be admitting her and when there is a room ready we will move. It's about 11 am before we get to our room, her fever is down and she has an IV running. I tell them she hasn't eaten in a few days and they have us try to give her some jello.

The same regular doctors that were there the first time come in to talk to us and I didn't realize how much better Primary Children's was until I wasn't there anymore. At PCMC you hit the call nurse button and a nurse is in your room before your finger comes off. Here you wait....and wait...and wait. It could be 10 minutes that your IV has been alarming before a nurse comes in on their own or you physically get up and go get them.
We discussed why we went to Utah, and what was recently going on with Hailey. Since we obviously knew Hailey has neurological issues and was unresponsive to us they wanted to do an EEG of the brain. She has never had one, but I did mention that I was curious as to why they never ordered one in Utah when we were there. I asked one of the neuro doctors on her team there and they said that in Hailey's MRI it's all white matter (or lack there of) that they were seeing issues with. Seizures occur in grey matter and there didn't seem to be an issue with that. Not knowing what to expect with an EEG we were all curious how Hailey would react to it. Like usual she was a champ. A little fussy with the probes being stuck all over, but nothing too major. When I watched the screen of the EEG and all the crazy lines all over the place I was sure there was going to be some thing new we would learn...luckily there wasn't. We were told that her EEG was consistent with a child that has leukodystrophy.

Then some blood came results came back. Her blood count came back at a 7. Normal is 12-15. When we left Utah her blood count was a 12. 5 days later it's at a 7 she is losing blood fast and A LOT of it. They wanted to know if her number was going up or down so we waited 12 hours to see, which I wasn't too happy about, but they had to know and we needed to start somewhere and 7 was our starting number.

Hailey was so pale. She usually has really pink chunky cheeks, her face was ghost white. They drew her blood at 3 am and at 8 am they came in to tell us her blood count was at a 6.2. She is still losing blood. At this point since she is litterally losing liters of blood, they want to make sure she isn't bleeding internally. There is no blood in her head, no blood in her urine and then they want to test her stool, but she hasn't eaten so how can she poop? They order an enema to go along with a Guaiac test. Where they will test her stool. *Side note: Could you imagine being the person who invented this test and then had it named after you? Kinda embarrassing* Anyway...
So they gave her the enema and then did the test. I remember the nurse and her CNA doing the test and saying, "that looks like a negative to me, all done." So no blood in her stool. Where did the blood go? We would have noticed if she was leaking liters of blood and I haven't seen any. When I talk to the doctor to ask, "now what?" They tell me they are waiting on the Guaiac test. Um...you already got the results it was a negative. Well, they couldn't find the results of the test anywhere. I guess the nurse didn't log it where they were looking for it and it took HOURS to confirm that the results were indeed negative. Oy! My head!
So I can ask again, "now what?" Well now we still have to figure out where the blood went. We talked to a hematologist, who was really great with Hailey and getting her to do what was asked of her. In the end we discover that Hailey had Hemolytic anemia associated with the IVIG treatment she had. It was a side effect that she had never had before, but was having now. She was going to need a blood transfusion. In order for that to happen she will need a PICC line placed. Yes...a PICC line. Like the one we JUST removed!! She will need to be put under and have it placed and that was going to happen this afternoon.
In my head I am not freaking out because I knew that she did well just a few days ago with it being put in and the placement takes about an hour and then she'll have the blood transfused. Well like usual things don't go as planned. We are taken up to the ICU and we wait for about an hour and a half before anyone even comes in to tell us that they are running late. She was supposed to have the PICC line placed at 2pm and they didn't get started until 4pm. Well when 5:30 rolled around and we had heard NOTHING, we start panicking. We ask the nurse to check and give us info. We are waiting in Hailey's room and at 6:00 the room phone rings and unfortunately I answer it. It was the Placement Team. The head doctor was on the phone and says, "we are unable to place the PICC line. Hailey is too dehydrated and we can't get a vein. We have tried repeatedly. It's urgent that she get the blood transfusion and the only other option is a central line." I have no idea what that means? I asked what that entailed and he said that it's like a PICC line but it's placed in the neck and they needed permission to do it...NOW. Well, crap. So I quickly explain to Josh what the heck is going on and we both know we weren't going to say no, stop, don't do it. When I ask how long more the procedure will be they tell me, it'll be about 15 more minutes, but Hailey is fine, breathing normal, oxygen is good. So I give consent and wait...again.
They tell us that we can go up and wait for her or that they will bring her down when she's done, but we go up and walk her to the room. They get the cold blood from the blood bank and there were a lot of signatures needed in order for it to start to flow. It was going to take nearly 4 hours for the whole transfusion. I just sat and watched in wonder. Who was so kind and generous to give their blood to my daughter?

It wasn't the prettiest time and it was pretty hard to get Hailey to be fully awake and smile. The line went straight into her neck and it looked so uncomfortable. She slept almost all night and the night doctor came in and said they will draw blood at 4 am and then every 8 hours to monitor her number.

The next morning Hailey was back and smiling. The color was back in her cheeks and her number was at a 10 by 7am, but she would continue to have her blood drawn through out the day. Her numbers kept going up little by little. After we had been there for 4 days and she was finally stable in terms of her anemia we get talking about the eating and drinking thing...

It's been over a week since we left Utah, and Hailey has barely eaten, but after that transfusion it's like she gets a burst of energy. She wants to eat it just looks like when I put food in her mouth she doesn't know what to do. She either keeps the food in there and does nothing with it, or she swallows over and and over and over. Either way she looks like eating is super uncomfortable for her and it's making me nervous. The attending on the floor says that it's time to think about maybe putting in a g-tube. That way Hailey can get the nutrition that she needs and if she can tolerate food again some day she could still take food by mouth. There are several issues. One, she has leukodystrophy and her team of doctors are in Salt Lake and we are in Las Vegas. Two, the PICC team said that her veins are traumatized and they don't think taking a good central line, that works perfectly well, out so that we can travel is a good idea. They can't discharge Hailey with the line in. It would take us over 8 hours to get to Utah if we drove without the line in. She wouldn't dehydrate in that short of time, but how would it go to put in another line if needed? The end solution to getting Hailey to Utah with the central line still in place was a hospital to hospital transfer via Life Flight. They would be able to have the line in, monitor Hailey and get us to Utah in about 2 hours. That was going to be our plan, but we needed to get it approved by insurance first. So we stayed at the hospital over the weekend and on Monday I was ready to say forget it. We could have been in Utah 2 days ago and she could have had her g-tube placed already. They said they just needed one more day for the medical board to review Hailey's case and to give us a yes or no. One more night at the hospital.
Tuesday morning we get the approval from insurance which I would have bet money on that I didn't think we would get. We did have to sign a ton of papers and say that we would pay if the insurance didn't.
Then we were told that the Vegas team got called out to Reno and don't know when they would be able to get us. When Primary Children's heard that we couldn't give them an ETA they arranged to come get us instead. Primary Children's to the rescue...again!

I am not good with motion. I get motion sick so easy. I always have. I was worried about going with Hailey. How would I take care of her and her needs if I am puking and can't take care of myself? So I took precautions. Josh bought me dramamine and these motion sickness wrist band pressure point things.

The flight team arrived and we were getting everything ready to go. Josh would drive to Salt Lake and meet us there late late that night. Hailey was in a good mood...but it wouldn't last.

Hailey and Lambie...ready for take off.

This was our plane. We had to take an ambulance from the hospital to the private airport. No celebrity sitings, just us...

In the above picture you can see Hailey having a morrow reflex with her arm out. Just going from an ambulance to an airplane would cause it and flying would be even worse...

Ignorance is bliss. This child did not know what we were in for. Everything started off fine. We took off, we were in the air for an hour and a half and then...we had to fly through a hail storm!! They told me it was going to get real bumpy for the next 20 minutes. They didn't tell me the plane would attempt to fall out of the sky several times. Hailey awoke the first time we dropped, reflexes going crazy. The second and third time we dropped she started getting sick. If we weren't strapped down, we'd be air born. She was lying down on her back and was unable to roll over to vomit. She was starting to choke on her vomit and they had to get the suction out and the hose had a kink and it didn't work. So the flight nurse is trying to scoop the vomit out of her mouth and fling it on the floor while the plane is shaking and dropping. I start to lose it. I didn't eat before we flew so I don't know where anything was going to come from, but it did. I started to vomit, Hailey was vomiting. The flight nurse is trying to help and I told him to take care of her. There was vomit ALL over. It ran down her neck into her central line, in her hair, in her clothes, on the blankets on lambie, on the floor...EVERYWHERE. I couldn't stop heaving. I just kept hearing, "hold on 15 minutes." "Just 10 more minutes." "5 Minutes we're going to be fine." It was the worlds longest 20 minutes of my life. I totally need a refund on the crap I bought to prevent that from happening.

When I could see ground I was relieved but still beyond motion sick. Hailey looked scared, I was scared. I was also so mad at Josh for saying that he would have wanted to be the one to fly with Hailey while I drove. After we land they transport Hailey to another ambulance and the flight crew had to clean out that plane and clean the puke off of everything before we could leave. I asked for a trash bucket and told the flight nurse to find the suction and get it ready but making sure that it worked this time.

The flight crew said that they had NEVER experienced such a rough flight. I told them that they will always remember my little Hailey then. I said it was our gift to them...LOL!

Once we arrived at the hospital there was a whirlwind of doctors and nurses ready to take care of Hailey. Poor thing was covered in vomit and was in pain since she threw up her Baclofen. The doctor gave an order for valium and we started to get Hailey all cleaned up. They took off her dressing that covered the central line and re-did all the tubing and put clean dressings on. They had orders for Hailey not to eat but start an IV and if she wanted to drink I had to add thickener to the liquid first. Well, Hailey was pretty out of it and I wished so bad that I had some of what she was having because I was going to have some serious PTSD after that ordeal. Around 11 pm I sneak out of the room and my stomach was wanting food even though my brain questioned it. I walked down to the cafeteria and thought out loud, "What are we doing back here? Why does Hailey have to go through all of this? There has to be an easy way out of all this. I got my food which just ended up being fries and a Diet Dr. Pepper and went back to the room. Josh got there about an hour later and we tried to figure out sleeping arragments. We didn't have a room at the Ronald House yet. I gave him the couch and I slept in a chair. Morning came way to fast.

Now in my head I am thinking, the team will come in already knowing that we are here for a G-Tube placement and we'll get the ball rolling with that and we'll be out of here is 3 days tops! The team came in and started talking about how they have a schedule set for an EEG, a swallow test, meeting with infectious disease, and an exome sequence test....Um? Where is place G-Tube in that list? Why all these other tests? You tell me last time that our daughter has pretty much the worst of the worst of diseases and that we need to enjoy the time we have left with her even though they can't tell us exactly how much time that is and now you want to do MORE tests?! They tell me that they just want to make sure that they are crossing everything off the list. If there is by some chance that Hailey has something else that presents like Leukodystrophy but that has a treatment they want to do that. Then on the other hand if that falls through you have to relive the leukodystrophy diagnosis...So then I bring up that Hailey literally just had an EEG like 4 days ago. Since it was done in Vegas they would feel better if they did the test themselves. They also do a more extensive test. The swallow test has to be done before you put a kid through surgery. To make sure that something that drastic is needed. So then why this Exome Sequencing test now. We were told last time we were here that the team decided against it. Well Hailey's case of have leukodystrophy isn't a common case. Usually when kids get diagnosed with it they just go downhill immediatly. There is no "getting better" with IVIG or Steroids. All those times she was in the hospital and went home and walked again and talked again, and ran and was better then she ever was should NOT have happened, does NOT happen. How did she go from completely unable to walk, talk, and bare weight to, running, jumping and speaking in complete sentences and then back down again. Right now she hasn't walked in over a month, hasn't spoken, hasn't been able to sit up, or eat or drink. Now it's happening too fast. With Exome sequencing they will need to test over 2500 of Hailey's genes. Then they also take my blood and Josh's too. They should be able to see where the "faulty" gene is from. We learn more about the Exome test and it ends up sounding like a bad fortune teller that only tells you bad things that will happen to you. If I have the gene for breast cancer it will tell us. If we have the gene for Huntington's Disease it will tell us. It will tell Josh and I that we have the gene for any specific diseases that there is most likely no cure for. So why did I sign the papers to want to know that?? I couldn't tell you. I guess first and foremost if there is something in that test that will help Hailey then I want to know. Second, if I do have a gene for a really crappy disease, well...I already have it. I can't get rid of it. Third, if we can find out if this is a gene that will show up again in one of our siblings or siblings children it would be helpful for them to know. They most likely won't do anything with that information but if it were me and I had heard there was a chance I would have a child born with a genetically fatal disease I would have NEVER had wanted to have children. So we signed the consent papers and with that you are agreeing to finding out the results...ALL the results, not just the ones that would be helpful for Hailey. The test comes back in 15 weeks and the anticipation of those results are going to give me an ulcer.
In the mean while they take Hailey's blood and the kids crew comes by with a project for Hailey and I to do together. Here is a pumpkin we painted together.

Josh thinks it's no big deal to get your blood drawn. He just sits there all calm and lets the lab tech do what she needs to do:

Me on the other hand who has had blood drawn before and has never enjoyed the experience, still freaks out over the anticipation of the needle piercing the skin...OUCH!!!

But it gets done and the blood is off for Exome Sequencing...I have yet to sit and think about how much this test is going to cost...

So now next on the list is the swallow study. We go down to the x-ray room and suit up in our lead aprons. They have different consistencies of food that they will be giving to Hailey while they record live x-rays of how it goes down. With pudding first Hailey swallows but the pudding only goes half way down which causes her to swallow repeatedly. It keeps going back up and she swallows and finally it goes down. Then with a nectar consistency, her brain doesn't tell her trachea to close so now the doctor on standby is ready for Hailey to aspirate. After the second try he calls off the test and says it's too dangerous to continue. In other words...She fails with flying colors.

They decide that until they can figure out if it is safe for Hailey to under go surgery they will place an NG or Nose Tube that will be giving Hailey food. It's something that the nurse does and It was not easy. First the have to measure Hailey to see how long the tube will have to be. Then they lube the tube so they can stick it up her nose and down her throat. In order for this to work, Hailey has to swallow at the exact right time. They try 3 times and finally it goes down with a swallow. It's bothersome and Hailey doesn't like it, but after 1/2 a day she is done trying to mess with it and they can begin feeds.

After the EEG, Haiely's hair was pretty goopy. The tech set up a inflatable bath so that we could wash Hailey's hair. She was also able to get her central line removed. When they did that they saw Hailey had developed adhesive burns on her neck.

Until we get word on the surgery, Hailey continues with Therapy. Here she is with occupational therapy. They were throwing bean bag animals down a barrel and I would catch them at the bottom. Hailey had a lot of fun!

Dr. Chung! He was on our neuro team, but his rotation had come to an end. He was a very compassionate doctor who answered every question we had and was always at the hospital. We could track him down in the hallway in the middle of the night and ask him to come look at Hailey and he always would. He was first in the room for rounds and he was always so sweet to Hailey. He is a great Doctor and when he told us his rotation was over and he was off to another part of the hospital we were truly sad that we wouldn't see him again. With a big hug and a picture with Hailey and Lambie we said goodbye. It was awesome meeting such a great doctor.

We finally get word that Hailey will indeed being having a G-tube placed the next morning. Hailey will be taken into surgery and a whole new chapter will be starting....